Background : Thalassemia is an inherited disease characterized by decreased production of globin chains. Two main treatments for thalassemia are routine blood transfusions and iron chelation therapy. However, many patients do not comply with the transfusions schedule. In this study, we want to explore about the benefit of maintaining high pre-transfusion hemoglobin level and the effect on iron deposition.
Material : This was a cohort retrospective study of 171 non-splenectomized children with thalassemia major (TM). This study was conducted in Cipto Mangunkusumo Hospital, January-December 2017. Mean pre-transfusion Hb, leukocyte, and thrombocyte level were observed for 1 year. Mean pre-transfusion Hb level was divided into high (HPTH, Hb level ?9 g/dL) and low (LPTH, Hb level <9 g/dL). The outcome of spleen size and body height were determined at the end of the study. The degree of iron deposition was assessed by T2*MRI.
Results : Mean age of the subjects was 13 years. The number of subjects between gender are equal. Most of them were diagnosed as ?-thalassemia (50.9%) or ?-thalassemia/HbE (46.8%). Around 65% of the patients consumed deferiprone as iron chelator. There was significant difference of spleen size between HPTH and LPTH group (p= 0.04). The prevalence of splenomegaly greater than Schuffner 2 was lower in HPTH subjects (11.3%), compared to LPTH subjects (33.9%). Large spleen size was associated significantly with thrombocytopenia (RR 5.02, p= 0.001), but not with neutropenia (p= 0.46). The number of stunted subjects between HPTH (34%) and LPTH (30.6%) was not differ significantly. There were extremely low positive correlations between mean pre-transfusion Hb level with cardiac and pancreas T2*MRI (r= 0.15 and 0.07).
Conclusions : As conclusion, many benefits can be obtained by maintaining pre-transfusion Hb level greater than 9 g/dL. The effect of iron deposition should not be worried if the patient complies with iron chelation therapy regimen.